lunatic fringe; beta-1,3-N-acetylglucosaminyltransferase lunatic fringe; O-fucosylpeptide 3-beta-N-acetylglucosaminyltransferase (LFNG)
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Function
- glycosyltransferase that initiates the elongation of O-linked fucose residues attached to EGF-like repeats in the extracellular domain of notch molecules.
- Lunatic fringe modifies Notch2 between the N-terminus & the 15th EGF repeat differing from manic fringe which modifies Notch2 between the 23rd & 29th EGF repeats thus resulting in a different modulation of Notch2 signalling
- decreases binding of JAGGED1 to NOTCH2
- does not effect binding of DELTA1
- mediator of somite segmentation & patterning (putative)
- transfers a beta-D-GlcNAc residue from UDP-D-GlcNAc to the fucose residue of a fucosylated protein acceptor
- soluble form may be derived from the membrane form by proteolytic processing (putative)
Structure
belongs to the glycosyltransferase 31 family
Compartment
- Golgi membrane
- single-pass type 2 membrane protein (putative)
Alternative splicing
named isoforms=3
Expression
- oscillations of lunatic fringe transcripts occur during somitogenesis controlled at the transcriptional level by clock elements in the promoter region of the gene
- Notch signalling activates lunatic fringe transcription but is not an inherent component of the oscillator
Pathology
- defects in LFNG are the cause of spondylocostal dysostosis type 3
More general terms
References
- ↑ Pourquie O. Vertebrate segmentation: lunatic transcriptional regulation. Current Biology 12:R699-R701, 2002 PMID: https://www.ncbi.nlm.nih.gov/pubmed/12401188
- ↑ Shimizu K et al. Manic fringe and lunatic fringe modify different sites of the Notch2 extracellular region, resulting in different signaling modulation. J Biol Chem 276: 25753-8, 2001. PMID: https://www.ncbi.nlm.nih.gov/pubmed/11346656
- ↑ Entrez Gene http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=3955